Amyloid Heart Disease

Abstract 

The systemic amyloidoses are an uncommon group of disorders characterized by the extracellular deposition of amyloid in one or more organs. Cardiac deposition, leading to an infiltrative/restrictive cardiomyopathy, is a common feature of amyloidosis. It may be the presenting feature of the disease or may be discovered while investigating a patient presenting with non-cardiac amyloidosis. In this article we review the features of cardiac amyloidosis and its varied manifestations. The need for a high index of suspicion and the critical importance of precise biochemical typing of the amyloid deposits is stressed in light of recent advances in therapy which can, when appropriately used, significantly improve prognosis.

Keywords: Amyloidosis, Biochemical typing, Cardiac deposition

Abbreviations and Acronyms: ACE, angiotensin-converting enzyme, Apo-A1, apolipoprotein A1, BNP, B-natriuretic peptide, ECG, electrocardiogram, IAA, isolated atrial amyloid, ICD, implantable cardioverter-defibrillator, MRI, magnetic resonance imaging, SSA, senile systemic amyloid, TTR, transthyretin